Probability of Remission of the Main Epileptic Syndromes in Childhood.

AIM: To determine the long-term probability of remission without antiepileptic treatment of common epileptic syndromes and of children without a specific syndromic diagnosis. PATIENTS AND METHODS: All children less than 14 years old with 2 or more unprovoked seizures seen at our hospital between June 1, 1994, and March 1, 2011 (n = 680), were included and prospectively followed up until August 15, 2020. Syndromic diagnosis was made retrospectively but blinded to subsequent evolution, employing the data available at 6 months after diagnosis and under predefined operational criteria. RESULTS: The Kaplan-Meier estimate of the probability of achieving a remission period of at least 5 years, with neither seizures nor antiepileptic treatment at 14 years was 97% for well-defined childhood epilepsy with centrotemporal spikes, 82% for uncertain childhood epilepsy with centrotemporal spikes, 85% for well-defined Panayiotopoulos syndrome, 88% for uncertain Panayiotopoulos syndrome, 93% for nonfamilial self-limited infantile epilepsy, 100% for familial self-limited infantile epilepsy, 86% for absence epilepsy, 6% for juvenile myoclonic epilepsy, 71% for cryptogenic West syndrome, 72% for patients with no associated neurologic deficits and no specific syndromic diagnosis, 65% for symptomatic West syndrome, and 40% for patients with associated neurologic deficits and no specific syndromic diagnosis. CONCLUSIONS: The study results highlight the long-term outcomes of the main epileptic syndromes and also of the patients with no syndromic diagnosis.

Frecuencia, semiología y pronóstico de la epilepsia infantil benigna / Frequency, semiology and prognosis of benign infantile epilepsy

Introducción. La epilepsia infantil benigna es un síndrome epiléptico sobre el que hasta ahora se ha publicado tan sólo un pequeño número de series de casos. Objetivo. Estudiar la frecuencia, semiología y pronóstico de la epilepsia infantil benigna. Pacientes y métodos. Los 827 pacientes con una o más crisis epilépticas no provocadas que consultaron en nuestro hospital entre el 1 de junio de 1994 y el 1 de marzo de 2011 fueron incluidos y seguidos prospectivamente. Se diagnosticaron de epilepsia infantil benigna los pacientes que cumplieron los siguientes criterios a los seis meses de evolución: una o más crisis focales o generalizadas, inicio antes de los 24 meses, ausencia de déficits neurológicos y electroencefalograma y neuroimagen normales. Resultados. Cumplieron los criterios diagnósticos 77 casos (9%). La semiología de las crisis fue similar a la de otras crisis focales en niños menores de 24 meses. Un 25% de los pacientes permaneció como con crisis aisladas. Entre los de dos o más crisis epilépticas, la probabilidad de alcanzar una remisión inicial de tres años sin tratamiento antiepiléptico fue del 86%. En el subgrupo de pacientes con crisis focales sin antecedentes familiares, la probabilidad fue del 74%, y en cinco casos se detectó posteriormente un retraso psicomotor o discapacidad intelectual. Conclusiones. La epilepsia infantil benigna es un síndrome epiléptico frecuente. La semiología de las crisis no es útil para caracterizar el síndrome. El diagnóstico de epilepsia infantil benigna a los seis meses de evolución implica un pronóstico razonablemente bueno, pero posiblemente no tanto como el de otras epilepsias autolimitadas de la infancia

Introduction. Benign infantile epilepsy is an epileptic syndrome of infancy. Until now, only a small number of case-series have been published. Aim. To study the frequency, semiology and prognosis of benign infantile epilepsy. Patients and methods. The 827 patients with one or more epileptic seizures seen at our hospital between 1 June 1994 and 1 March 2011 were included and prospectively followed. A diagnosis of benign infantile epilepsy was made in patients that fulfilled the following criteria at six month of evolution: one or more focal and/or generalised seizures, onset before 24 months, no neurological deficit and normal neuroimaging and interictal EEG. Results. 77 cases (9%) met the diagnostic criteria. Semiology of the seizures was similar to that of other focal seizures in children under 24 months. 25% of the patients remained as isolated seizures. Among those with two or more seizures, the probability of achieving a 3 year initial remission without antiepileptic treatment was 86%. In the subgroup of patients with focal seizures without family history the probability was 74% and in five cases a global developmental delay/intellectual disability was detected thereafter. Conclusions. Benign infantile epilepsy is a frequent epileptic syndrome. Semiology of seizures is not useful to characterize the syndrome. A diagnosis of benign infantile epilepsy at six month of evolution implies a reasonably good prognosis, but possibly not as good as for other self-limited epilepsies of infancy

A study of drug-resistant childhood epilepsy testing the new ILAE criteria.

PURPOSE: To test the new ILAE definition of drug-resistant epilepsy in a cohort study. METHODS: All children younger than 14 with two or more unprovoked seizures observed at our hospital between 1994 and 2008 were included. RESULTS: Five hundred and eight patients were followed for an average of 90 months (range 24-168). The probabilities of achieving seizure freedom, according to the ILAE criteria, with the first, second, third and fourth and subsequent therapeutic regimens were 65%, 29%, 27% and 21%, respectively. In the cohort, 87 patients met the criteria for drug-resistant epilepsy, which represents 19% of the treated patients (n=459) and 17% of the overall sample. The probability of meeting the criteria for drug-resistant epilepsy was 11%, 11% and 13% at 2, 6 and 10 years respectively. Sixty two percent of drug resistant cases were younger than 4 years old, 73% had an associated developmental delay and/or motor deficit, 42% had an identifiable structural cause of epilepsy and 32% had a specific epileptic syndrome. For drug-resistant patients who tried additional therapeutic regimens, the probability of achieving a seizure-free state without further recurrences was 23% and 27% at three and five years, respectively. CONCLUSIONS: Compared with more stringent criteria, the new ILAE criteria classify a greater number of patients with drug-resistant epilepsy. A significantly higher proportion of cases meeting this definition subsequently enter remission. A definition of drug-resistance that includes the additional criteria of failure of a third antiepileptic drug or high seizure frequency may better identify patients with truly drug-resistant epilepsy.